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The urea cycle is the final pathway for nitrogen metabolism. Urea cycle disorders (UCDs) include a variety of genetic defects, which lead to inefficient urea synthesis. Elevated blood ammonium level is usually dominant in the clinical pattern and the primary manifestations affect the central nervous system. Herein, we report the case of a 17-year-old girl who was diagnosed with UCD at the age of 3. Despite a controlled diet, she was hospitalized several times for acute attacks with recurrent life risk. She came to our attention for a hyperammonemic episode. We proposed an orthotopic liver transplant (OLT) as a treatment; the patient and her family were in complete agreement. On February 28, 2007, she successfully received a transplant. Following the surgery, she has remained well, and she is currently leading a normal life. Usually for UCDs diet plays the primary therapeutic role, while OLT is often considered as a last resort. Our case report and the recent literature data on the quality of life and prognosis of traditionally treated patients vs OLT patients, support OLT as a primary intervention to prevent life-threatening acute episodes and chronic mental impairment.

Background: Tuftsin activity (TA) is reduced in cirrhosis. This contributes to the defective phagocytic activity (PA) of neutrophil granulocytes and is related to the impairment of splenic function. Orthotopic liver transplantation (OLT) cures cirrhosis and might restore TA. This study was aimed at determining if OLT restores TA and PA., Methods: We measured in 9 cirrhotic patients, before and after successful OLT, TA by a bioassay and PA by chemiluminescence in which neutrophils of the patient were tested with both autologous (PA1) and pooled sera from healthy subjects (PA2). Splenic function was assayed by the pitted red cell count., Results: Before OLT, TA was reduced in 7 patients, and PA1 in all the patients. Pitted cell count was elevated in all the patients. After OLT (median 39 months; range 21-49), TA improved in all cases [median: from 8% (5-16%) to 20% (9-22%), p < 0.008], normalizing in 5 out of the 7 patients with low values. PA1 improved in all the patients [from 102 cpm (65-128 cpm) to 235 cpm (78-280 cpm), p < 0.008], normalizing in 5. Pitted red count decreased in 7 patients and normalized in 3 [from 3.3% (2.1-6.0%) to 2.4% (1.4-2.8%), p < 0.021]. Platelet count [from 55 x 10(3) (30-100) to 185 x 10(3) (93-286), p < 0.008] and leucocyte count [from 3.60 x 10(3) (1.81-5.23) to 5.53 x 10(3) (3.31-6.71), p < 0.008] also improved., Conclusions: OLT improves TA and PA of cirrhotic patients. This effect is associated with an improvement of both functional hyposplenism and haematological hypersplenism. The restoration of natural defences against infections may mitigate the adverse effect of immunosuppressive treatment., (Copyright 2005 S. Karger AG, Basel)

Background/aims: Liver transplantation for alcoholic end-stage liver disease remains controversial at many transplant centers. The aim of the present study was to evaluate the outcome of patients with alcoholic liver disease who underwent liver transplantation at Centro Trapianti, Policlinico S. Orsola, Bologna., Methodology: We describe the outcomes of 18 alcoholic patients with end-stage liver disease who received orthotopic liver transplants at our center from April, 1986 to February, 1996. The data obtained was compared with that of 114 patients with virus-related cirrhosis selected as transplant controls. An absolute period of abstinence from alcohol consumption for at least six months was required., Results: Regarding the actuarial survival rate and non-fatal post-transplant complications, no significant differences were noted in comparing the non-alcoholic with the alcoholic recipients, except for a higher incidence of Cytomegalovirus infection in the alcoholic group followed-up for more than four months. The alcoholic relapse rate was 27.2%, but only one patient returned to harmful drinking. Seventy-three percent of subjects who were followed-up for at least six months were occupied in gainful employment. Alcoholic relapse did not affect employment status., Conclusion: This data demonstrates that liver transplantation for selected patients with end-stage alcohol-related cirrhosis achieves good results in survival, complications and employment status, and it appears difficult to defend an inflexible claim to have demonstrated an absolute long-term abstinence before transplantation when a severe deterioration of liver function is present.

We monitored the immune response after liver transplantation in 20 patients by measuring the serum levels of soluble interleukin-2 receptor (sIL-2R), soluble CD8 (sCD8), serum amyloid-A protein (SAA), and tumor necrosis factor-alpha (TNF-alpha). In six patients data were available to extend the follow-up period to one year. In all patients mean sIL-2R levels increased in the first month after liver transplantation, and subsequently decreased to values similar to pre-OLT ones, while SAA mean levels rose in the first week after OLT only in patients with rejection. sCD8 levels did not significantly rise after OLT, and TNF-alpha was undetectable in most cases. During episodes of rejection, rejector patients had significantly higher levels of sIL-2R, sCD8, and SAA than stable (without complications) patients. Conversely, no significant difference between rejectors and patients with other complications existed for any of the markers studied. These results diminish the importance of these serum markers of immune activation as laboratory tools in the differential diagnosis of acute rejection from other complications. However, sIL-2R, SAA, and sCD8 levels correlated with the histological grade of rejection and therefore can be utilized to monitor patients with an established diagnosis of acute rejection.

The authors report their experience with angiographic techniques in the diagnosis of vascular complications after liver transplantation. From 1986 to 1990, 78 patients were transplanted in our Hospital; of them, 8 underwent angiographic investigations for vascular complications. Angiography is very important when vascular complications are suspected, in the patients with a rise in cytolytic enzymes and in bilirubine levels, with hyperpyrexia, and with bioptic confirmation of no rejection. Duplex US is useful in the evaluation of portal canalization; if there are any doubts, angiography is performed also in the preoperative phase.

Primary graft non-function remains one of the most life-threatening problems after liver transplantation. Its etiology is still unclear. Liver retransplantation is the only therapeutic alternative to this problem. The PGE1 series of prostaglandins have recently been successfully used in the treatment of transplanted organ dysfunction. In this paper we describe a case of primary graft non-function successfully treated with continuous infusion of prostaglandin PGE1, and we briefly discuss the pathogenetic and therapeutic hypotheses.